Congenitally corrected transposition of the great arteries in the adult: functional status and complications.

OBJECTIVES: We sought to assess the clinical outcome, functional status and complications of adult patients with congenitally corrected transposition of the great arteries.

BACKGROUND: Congenitally corrected transposition is a rare form of congenital heart disease, although survival into adult life may be expected. Little information is available on the long-term prognosis of these patients once they have reached adulthood. This study focuses exclusively on patients >18 years old followed up at a single tertiary referral center.

METHODS: The charts of all patients with a diagnosis of congenitally corrected transposition of the great arteries from the Toronto Congenital Cardiac Centre for Adults since 1985 were reviewed. Data were available for 52 patients, 26 of whom had undergone radionuclide angiography. Mortality, clinical and functional status, surgical procedures and complications were reviewed.

RESULTS: Thirteen patients (25%) died; age at death was 38.5 +/- 12.5 years (mean +/- SD). The current age of survivors is 32.7 years (range 18.2 to 54.3). Of the survivors, 17 had palliative procedures, and 25 had definitive repair, 11 of whom required reoperation. Left ventricle to pulmonary artery conduit replacement was necessary in seven patients. Eighteen patients have permanent pacemakers, nine of whom developed complete heart block perioperatively. Nine patients developed progressive atrioventricular (AV) block unrelated to operation. Supraventricular arrhythmias occurred in 15 patients. Progressive systemic AV valve regurgitation developed in 10 patients and endocarditis in 6.

CONCLUSIONS: Congenitally corrected transposition in the adult patient is not a benign condition. Late complications are common and warrant careful, long-term follow-up.